Extrahepatic portal hypertension following liver transplantation: a rare but challenging problem

This study reports our experience of 8 cases of extrahepatic portal hypertension after 273 orthotopic liver transplantations in 244 adult patients over a 10-year period. The main clinical feature was ascites, and the life-threatening complication was variceal bleeding. Extrahepatic portal hypertension was caused by portal vein stenosis in 6 patients, and left-sided portal hypertension in 2 patients after inadventent ligation of portal venous tributaries or portasystemic shunts. All patients with portal vein stenosis had complete relief of portal hypertension after percutaneous transhepatic venoplasty (n = 4) or surgical reconstruction (n = 2), after a median follow-up of 33 (range: 6-62) months. Of the 2 patients with left-sided portal hypertension, one died after splenectomy and one rebled 6 months after left colectomy. This study suggests that extrahepatic portal hypertension is a series complication after liver transplantation that could be prevented by meticulous portal anastomosis and closure of portal tributaries or portasystemic shunts to improve the portal venous flow. However, any ligation has to be performed under ultrasound guidance to avoid inadventent venous ligations.     

Hepatic and portal surface veins: a new anatomic variant revealed during abdominal CT

OBJECTIVE: The objective of this paper is to describe a new finding on CT of hepatic and portal vein segments located in a subcapsular location on the surface of the liver. SUBJECTS AND METHODS: From a series of more than 11,000 contrast-enhanced abdominal CT scans performed from 1993 to 1997, 14 patients were identified as having hepatic or portal vein segments or both in a subcapsular location on the surface of the liver. RESULTS: We found seven portal vein surface segments in seven patients and 14 hepatic vein surface segments in 12 patients. Of the 14 patients, five had both portal and hepatic vein surface segments. Therefore, in a cohort that exceeded 11,000 patients, the incidence of this finding was 0.1%. Four patients had cirrhosis, two had small hypervascular liver lesions, and eight had healthy livers. The surface veins were not associated with any other recognized vascular anomalies or with anastomoses to extrahepatic systemic veins. CONCLUSION: Hepatic and portal veins can course to a subcapsular location on the surface of the liver. This anatomy is believed to be a normal variant and can be found in patients with healthy livers and normal hepatic vein hemodynamics and in patients with portal hypertension.     

Intrasplenic venous flow patterns demonstrated by Doppler ultrasound in patients with portal hypertension

The flow pattern in the splenic vein has been previously reported in patients with portal hypertension, but with no reference to the flow within the intrasplenic venous radicles. Using Doppler ultrasound, this study describes the intrasplenic venous flow direction in 176 adult patients with intrahepatic portal hypertension. In our series, a normal flow pattern was maintained in all except four patients (2.3%) who had either reversed or dual venous drainage patterns resulting in trans-splenic portosystemic shunts. These abnormal patterns are Doppler signs of portal hypertension which might be associated with a higher risk of oesophageal variceal bleeding. It is recommended that the intrasplenic venous flow pattern should be assessed before surgical intervention involving the spleen in patients with portal hypertension.     

Recanalized umbilical vein in portal hypertension

Experience with splenoportography suggests that patency of the umbilical vein occurs in about 9% of the patients with portal hypertension. A widely patent umbilical vein might serve as a decompressive portosystemic shunt. Percutaneous transhepatic portography was performed in 107 patients with cirrhosis of the liver and portal hypertension. A patent umbilical vein was found in 28 patients (26%). This finding significantly paralleled the number and size of other collateral veins, apart from gastroesophageal varices. No significant relation was found between umbilical vein patency and portal pressure, extrahepatic shunting, variceal bleeding, or ascites. It is concluded that a large patent umbilical vein does not effectively relieve portal hypertension, prevent gastroesophageal varices, or protect against variceal bleeding or ascites.     

The transjugular stent implantation for the treatment of malignant portal and hepatic vein obstruction in cancer patients

BACKGROUND: The increase in portal vascular resistance is a significant complication of metastatic disease to the liver or locally advanced cancer, e.g., biliary cancer. PATIENTS AND METHODS: This paper describes the successful palliative treatment of two cancer patients with portal hypertension presenting with the symptoms of tense ascites, mesenteric congestion, and severe variceal bleeding. By creating a stenttract between a hepatic vein and a main branch of the portal vein and/or by placing an extendable stent into the portal vein, the transjugular intrahepatic portosystemic stent-shunt (TIPS) technique was used to decompress the portovascular system. RESULTS: The TIPS-technique offers a new, safe and effective palliation for malignant portal hypertension. In both patients, the symptoms of the portal hypertension disappeared after the procedure. This was accompanied by a significant improvement of the patients performance status allowing an early ambulation. CONCLUSION: Our findings demonstrate the feasibility and effectiveness of the TIPS procedure as a minimal invasive treatment for portal vein decompression in selected tumor patients.     

Pulmonary hypertension associated with portal hypertension. Apropos of 2 cases

The authors report the cases of two patients with pulmonary hypertension associated with portal hypertension. This is a rare association with a reported prevalence ranging from 0.25 to 0.73%. The diagnosis of portal hypertension preceded that of pulmonary hypertension by several years. The physiopathological mechanism of the latter is not well known although several hypotheses have been proposed. Treatment is only symptomatic. The prognosis is usually poor, the causes of death being related to complications of liver failure and/or portal hypertension or to those of pulmonary hypertension.     

Effect of losartan, an angiotensin II receptor antagonist, on portal pressure in cirrhosis

Administration of angiotensin II causes an increase in portal pressure, and plasma concentration of angiotensin II is elevated in patients with cirrhosis, suggesting that angiotensin II may be involved in the pathogenesis of portal hypertension in cirrhosis. We evaluated the effect of the orally active angiotensin II receptor antagonist, losartan, on portal pressure in patients with cirrhosis and portal hypertension. Thirty patients with severe (hepatic venous pressure gradient [HVPG] >/= 20 mm Hg) and 15 patients with moderate (HVPG < 20 mm Hg) portal hypertension at baseline measurement were treated with an oral dose of 25 mg losartan once daily for 1 week and compared with 15 (HVPG >/= 20 mm Hg) and 10 (HVPG < 20 mm Hg), respectively, cirrhotic controls. On the seventh day, HVPG was determined again, and blood pressure, heart rate, body weight, and parameters of liver and kidney function were recorded. Losartan induced a significant (P <.001) decrease of HVPG in the patients with severe (-46.8% +/- 15.5%) and moderate (-44.1% +/- 14.7%) portal hypertension, while no significant change was seen in the controls. Losartan caused a slight but significant (P <.01) fall in mean arterial blood pressure (-3.1 +/- 5.0 and -3.5 +/- 4.3 mm Hg, respectively). One patient treated with losartan had a short symptomatic hypotensive reaction after the first dose of losartan that did not recur despite continued treatment. No deterioration of liver or kidney function was observed. The present study indicates that angiotensin II blockade with orally administered losartan is safe and highly effective in the treatment of portal hypertension.     

Pulmonary vascular disorders in portal hypertension

The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance is elevated. Since hepatopulmonary syndrome and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the common factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmonary syndrome seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of portopulmonary hypertension. Resolution of hepatopulmonary syndrome is common after liver transplantation, which has an uncertain effect in portopulmonary hypertension. The pathophysiology of both syndromes may involve vasoactive mediators and angiogenic factors.     

Changes in lymph vessels and portal veins in the portal tract of patients with idiopathic portal hypertension: a morphometric study

Little is known about the effects of the pathological process associated with idiopathic portal hypertension (IPH) on hepatic lymph vessels or lymph flow. We used morphometric analysis to examine IPH-associated changes in lymph vessels and branches of the portal vein, with use of immunohistochemical staining for alpha smooth muscle actin. We also quantitated these changes using an image analysis system. The study was conducted with use of liver wedge biopsy material from 10 patients with advanced IPH and 10 control samples from patients with gastric carcinoma without liver disease. The number of lymph vessels, identified by a lack of smooth muscle layer in the wall, and the ratio of the total area of these vessels to that of the portal tract were higher in IPH samples than in the control samples, but the ratio of the area of a single lymph vessel to that of the portal tract in IPH samples was not different from control samples. The number of portal vein branches, characterized by hypertrophy of the smooth muscle layer in IPH samples was not different from control samples. The ratio of the total area of these branches to that of the portal tract, and the ratio of a single portal vein branch to that of the portal tract, were lower in IPH samples than in the control samples. Our results suggest that these morphometric changes in IPH may be associated with a reduction in portal blood flow and increased lymph flow, and that the latter may in turn reduce the high portal vein pressure in idiopathic portal hypertension.     

Portal hypertensive colopathy: histologic appearance of the colonic mucosa

BACKGROUND/AIMS: The aim of this study was to evaluate macroscopic and histologic disorders of the colon in rats with portal hypertension. METHODOLOGY: The animals were divided into two groups: 10 rats in the experimental group and 10 controls. Portal hypertension was induced by a partial ligation of the portal vein. We assessed the histologic appearance and macroscopic alterations of the colonic mucosa 12 weeks after the induction of portal hypertension. RESULTS: The macroscopic results showed the presence of the typical signs of portal hypertension, with dilatation and tortuosity of the mesenteric veins and hyperemia of the rectum and colon. Histologic study revealed a marked dilatation in the microcirculation at the level of the ascending colon (20 +/- 2 micra in diameter) and rectum (30 +/- 4), with a tendency to group the lesions. Submucosal edema without inflammatory signs was observed. CONCLUSION: The existence of these lesions in the colon and rectum can be the cause of hemorrhage, as in the gastropathy of portal hypertension and should be considered in cases of undiagnosed hemorrhage in patients with portal hypertension.     

Optimal recovery of cytomegalovirus from urine as a function of specimen preparation

BACKGROUND/AIMS: Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension. Some severe pulmonary vascular anomalies associated with portal hypertension disappear after isolated liver transplantation. Evolution of pulmonary artery hypertension due to plexogenic arteriopathy is controversial, as this association is still considered a contraindication to isolated liver transplantation. Outcome of pulmonary hypertension after isolated liver transplantation is reported in three patients with portal hypertension. METHODS: After echocardiographic diagnosis, the patients had a complete hemodynamic exploration, and two had a lung biopsy. After liver transplantation, the survivors had echocardiographic follow up and a second hemodynamic exploration. RESULTS: In two children, pulmonary pressures and resistances returned to near-normal values 1 and 6 years after successful isolated liver transplantation. The third patient, with the most severe arteriopathy, had to wait 1 year for a donor, and the attempted transplantation was complicated by ventricular tachycardia; death occurred 2 days after surgery. CONCLUSIONS: Liver transplantation can reverse pulmonary artery hypertension due to high pulmonary resistances complicating liver disease with portal hypertension, provided it is carried out at an early stage. Early detection of pulmonary hypertension by systematic echocardiography may thus be crucial in these children with portal hypertension.     

Haemodynamic assessment

The degree of portal hypertension and its haemodynamic complications can be easily determined. However, the interpretation of these values is not entirely clear and further clinical and experimental studies are needed to explain why some patients with portal hypertension bleed from oesophageal varices while others do not.     

The postprandial portal flow is related to the severity of portal hypertension and liver cirrhosis

BACKGROUND/AIMS: Diminished postprandial portal hyperemia has been demonstrated by echo-Doppler flowmetry in patients with liver cirrhosis, but its diagnostic role is unclear. This prospective study was therefore undertaken in patients with varying severity of portal hypertension and degree of liver cirrhosis. METHODS: Portal flowmetry was performed in 66 patients with cirrhosis and 20 healthy volunteers during fasting and 30 min after ingestion of a standardized meal. Hemodynamic parameters were related to the degree of esophageal varices, variceal bleeding, portal hypertensive gastropathy and Child-Pugh score. RESULTS: The postprandial portal blood velocity increment was low in patients with esophageal varices of any degree (22-24%), compared to patients without varices (49%, p<0.01) and healthy controls (65%, p<0.001), but was not different in patients with or without variceal bleeding (22% vs. 20%). In contrast, the congestion index (CI; ratio of portal vein cross-sectional area and portal blood velocity) pre-/postprandial decreased in the bleeding group only (CI pre/ CI post 1.30+/-0.23 (no bleeding) vs. 0.86+/-0.29 (bleeding); p<0.01). Portal hypertensive gastropathy was not related to any of the portal flow parameters. The portal blood velocity increment was comparable in controls (65%) and patients with Child-Pugh class A cirrhosis (56%), but lower in patients with class B (32%) and class C cirrhosis (15%, p<0.05 vs. class A). Also, there was no postprandial decrease in congestion index in patients with the most severe cirrhosis (p<0.01 class C vs. class A and B). CONCLUSIONS: The postprandial rise in portal flow is inversely related to the severity of portal hypertension and liver cirrhosis, and may be a valuable parameter with respect to the risk of variceal bleeding.     

Endosonographic, endoscopic, and histologic evaluation of alterations in the rectal venous system in patients with portal hypertension

BACKGROUND: Colorectal varices and congestive rectopathy or colopathy have been erratically reported in patients with portal hypertension. The clinical importance of these entities has not been described. We assessed the changes in the venous system of the rectum by endoscopy and rectal endosonography (EUS). We also assessed the role of factors such as etiology of portal hypertension, grade of esophageal varices, sclerotherapy, and liver disease severity on the occurrence of these vascular changes. METHODS: We studied changes in the venous system of the rectum using endoscopy and EUS in 60 patients with portal hypertension (cirrhotic 41, noncirrhotic 19). Ten patients with irritable bowel syndrome and 6 patients with hemorrhoids served as controls. Rectal varices were classified as tortuous, nodular, and tumorous. Corresponding appearances on rectal EUS were classified as single or discrete multiple, multiple, and innumerable submucosal veins, respectively. Evidence of congestive rectopathy was also recorded. RESULTS: Prevalence of rectal varices was 43.3% on endoscopy (73% tortuous, 19% nodular, and 8% tumorous) and 75% on EUS (p < 0.0005). The latter showed corresponding appearances of submucosal veins in 25 of 26 patients and detected submucosal veins not identified at endoscopy in 19 other patients. Congestive rectopathy was found in 38.3% of patients. Multiple small dilated vessels in the submucosa were seen in 23.3% patients on rectal EUS. The development of these vascular changes was significantly influenced by sclerotherapy, but not by higher grade of esophageal varices, the etiology of portal hypertension, or severity of liver disease. CONCLUSIONS: Changes in the rectal venous system are common, with rectal EUS being superior to endoscopy in detecting early, as well as florid, changes.     

Haemodynamic changes in portal hypertension: new insights in the pathogenesis and clinical implications

In man and experimental animals, portal hypertension with portal-venous collaterals, is associated with a hyperdynamic circulation, caused by peripheral vasodilatation, mainly in the splanchnic bed. This peripheral vasodilatation is clinically important, since it is thought to be responsible for the pathogenesis of complications of portal hypertension such as ascites, the hepatorenal syndrome and portal hypertensive gastropathy and colopathy. Many cirrhotic patients may not die primarily because of their hepatic dysfunction, but rather because of the consequences of the circulatory abnormalities which are secondary to the liver disease. Circulating hormonal vasodilators from intestinal origin such as glucagon, insufficiently cleared by the liver, are only partly responsible for these changes. Recent experimental data point to a role for an increased production of the locally acting potent vasodilator nitric oxide in the vascular wall, in the pathogenesis of the hyperdynamic circulation. Furthermore, nitric oxide seems to play an important role in the development of portal-venous collaterals. Modulation of the nitric oxide production might offer therapeutic options for the treatment of portal hypertension and its complications.     

Longterm outcome after injection sclerotherapy for oesophageal varices in children with extrahepatic portal hypertension

A consecutive series of 36 children with bleeding from oesophageal varices secondary to extrahepatic portal hypertension was successfully treated by endoscopic injection sclerotherapy and followed up over a mean period of 8.7 years after variceal obliteration. There were no deaths from portal hypertension or its treatment and morbidity related to oesophageal sclerotherapy was minimal. Endoscopic injection sclerotherapy alone proved safe and effective in controlling variceal bleeding from portal hypertension in over 80% of the children. Recurrent variceal bleeding developed in 10 (31%) patients but half of these were effectively treated by further sclerotherapy. Gastric variceal bleeding unresponsive to sclerotherapy necessitated successful portosystemic shunt surgery in four (13%) patients. Two children required splenectomy for painful splenomegaly. In most children injection sclerotherapy is the best treatment for the primary management of bleeding oesophageal varices, reserving portosystemic shunting or other surgical procedures for those with bleeding from gastrointestinal varices.     

Hypersplenism in surgery

We studied 86 patients suffering from hypersplenism, associated with portal hypertension (39 patients), hematologic disease (45 patients) and with Gaucher's disease (2 patients). Inclusion criteria were the presence of thrombocytopenia < 100.000 x mm3 and leucopenia < 4.000 x mm3. No meaningful differences about the improvement of hypersplenism in portal hypertension were observed between the patients submitted to shunt and splenectomy and those submitted to shunt only. On the contrary, splenectomy caused a quick normalization of the leukothrombocytopenia in the patients suffering from hematologic disease, which, moreover, allowed to perform radio- and chemotherapy in those suffering from malignancies. We reported absence of mortality and a low and aspecific morbidity.     

Choledochal cyst: a review of 19 cases

Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.     

Portal-hepatic venous shunt through a portal aneurysm complicated by hepatic encephalopathy and pulmonary hypertension

We report a rare case of portal-hepatic venous shunt through an enormous portal aneurysm complicated by pulmonary hypertension. A 66-year-old woman was admitted to our hospital for hepatic encephalopathy. Chest roentgenography revealed pulmonary hypertension. Computed tomography and ultrasound examination demonstrated a shunt between the portal and hepatic veins through an enormous portal aneurysm. The diagnoses of portal-hepatic venous shunt and pulmonary hypertension were confirmed by hepatic venous catheterization and cardiac catheterization. Pulmonary hypertension might result from the effects of vasoconstrictive agents, which should be metabolized by the liver in normal subjects, passing through the intrahepatic shunt into the lung.     

Anomalous junction of the pancreaticobiliary duct accompanied by gallbladder cancer and obstructive jaundice in a patient with high serum and bile cytokine levels

BACKGROUND: Decompression of extrahepatic portal hypertension by directly bypassing the thrombosed portal vein has never been reported in cases of children with idiopathic (or neonatal) portal vein obstruction and cavernoma. METHODS: Seven children (15 years or younger) with portal vein obstruction requiring surgical decompression (urgently in two cases), and in whom preoperative Doppler had shown that the intrahepatic portal branches were hypoplastic but free of thrombus, were included in a pilot study. The cavernoma was bypassed by interposing a venous jugular autograft between the superior mesenteric vein and the distal portion of the left portal vein. Patients received follow-up using routine clinical parameters, upper gastrointestinal endoscopy, and Doppler ultrasound. RESULTS: The mesenterico-portal bypass restored a direct (physiological) hepatopetal portal flow. The operation resulted in effective portal decompression as demonstrated by decrease of the pressure gradient, rapid regression of clinical signs of portal hypertension, and definitive control of bleeding. CONCLUSIONS:This study shows that direct bypassing of portal cavernoma is possible and results in effective portal decompression. Restoration of the hepatic portal flow is a major advantage compared with conventional surgical shunting procedures. This new technique is potentially applicable to two thirds of children with portal vein thrombosis and should be considered when shunting procedures are indicated.